A case report of Scleral Buckle implant mimicking an orbital tumor

Objective@#We report a case of a scleral buckle mimicking an orbital tumor 28 years after a retinal detachment repair.@*Methods@#This is a case report.@*Results@#A 75-year-old male consulted for progressive, painless blurred vision of the right eye. He had a history of scleral buckling surgery for retinal detachment on the right eye in 1990 that restored his vision. Examination showed right eye ophthalmoplegia and inferior displacement of the globe. Imaging revealed a right supero-temporal orbital mass. Excision of the encapsulated mass was performed. Histopathology revealed an acellular, amorphous, granular and eosinophilic material with no evidence of malignancy. These were consistent with a foreign body. Postoperatively, there were improvements in ocular motility and hypoglobus.@*Conclusion@#In patients who present with limited ocular motility and have undergone scleral buckling, hydrogel scleral buckle overexpansion should be considered. Excision of such implants is warranted to resolve the signs and symptoms and confirm the etiology.

Differentiating intradiploic orbital dermoid and epidermoid cysts utilizing clinical features and machine learning

Purpose: The purpose of this study was to characterize intradiploic dermoid and epidermoid orbital cysts to determine any differences in clinical, radiographic, or surgical features. Methods: A retrospective review was performed of patients presenting with intradiplopic dermoid or epidermoid cysts. Additionally, a complete review of the literature was performed to identify cases of intradiplopic orbital dermoid and epidermoid cysts. Data collected included age, sex, presenting symptoms, location of intradiplopic cyst, ophthalmic findings, treatment, and follow?up. Clinical features of dermoid versus epidermoid cyst were compared. Additionally, machine?learning algorithms were developed to predict histopathology based on clinical features. Results: There were 55 cases of orbital intradiploic cysts, 49 from literature review and six from our cohort. Approximately 31% had dermoid and 69% had epidermoid histopathology. Average age of patients with dermoid cysts was significantly lesser than that of patients with epidermoid cysts (23 vs. 35 years, respectively; P = 0.048). There was no difference between sex predilection, presenting symptoms, radiographic findings, or surgical treatment of dermoids and epidermoids. The majority of patients (64%) underwent craniotomy for surgical removal. Machine?learning algorithms KStar and Neural Network were able to distinguish dermoid from epidermoid with accuracies of 76.3% and 69%, respectively. Conclusion: Orbital intradiploic cysts are more commonly epidermoid in origin. Dermoid cysts presented in younger patients; however, there were no other significant differences in features including ophthalmic or radiographic findings. Despite similar features, machine learning was able to identify dermoid versus epidermoid with good accuracy. Future studies may examine the role of machine learning for clinical guidance as well as new surgical options for intervention.

Large orbital glial heterotopia- A rare entity

A 15-month-old child was brought to the ophthalmology outpatient department with his parents complaining of a large mass located on the nasal side of his right eye since birth. On examination, an irregular mass was seen to be located on the nasal side of his right orbit, obscuring the visual axis. On a contrast-enhanced computed tomography scan, a heterogenous lesion was seen to be located on the bridge of the nose and the medial aspect of the right orbit without any bony defect or communication with the brain or sinuses. In view of the possibility of developing amblyopia, the lesion was surgically removed. On histopathological examination, a diagnosis of extranasal glial heterotopia was made. To the best of our knowledge, this is the largest extranasal glial heterotopia in the orbit to be ever reported.

Application value of the contrast-enhanced ultrasound in the diagnosis of orbital retrobulbar tumors / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To analyze the characteristics of the contrast-enhanced ultrasound in orbital retrobulbar tumors, and to explore the application value of the contrast-enhanced ultrasound in the diagnosis of orbital retrobulbar tumors.<p>METHODS: The contrast-enhanced ultrasound data of the 52 patients(52 eyes)with orbital retrobulbar tumors in the Fourth People's Hospital of Shenyang from March 2012 to May 2015 were retrospectively analyzed. According to the pathological results, 41 eyes were divided into benign group and 11 eyes were malignant group. The time intensity(TI)curve and quantitative data were obtained by SonoLiver software.<p>RESULTS: The TI curve of benign group mainly showed fast in and slow out mode, while that of malignant group mainly showed fast in and fast out mode. Quantitative data: rise time(RT)and mean transit time(mTT)in malignant group were lower than those in benign group, while there was no significant difference in maximal intensity(IMAX)between the two groups(<i>P</i>>0.05).<p>CONCLUSION: Contrast-enhanced ultrasound can provide imaging basis for the differential diagnosis of orbital retrobulbar tumors which is helpful for the preoperative qualitative diagnosis.

Clinical imaging analysis of 12 cases of orbital solitary fibrous tumor / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM:To investigate the characteristics of color Doppler sonography and MRI in orbital solitary fibrous tumor(SFT). <p>METHODS: It was a retrospective case series study. A total of 12 cases of orbital solitary fibrous tumors were recruited from April 2013 to August 2018 in Tianjin Medical University Eye Hospital. Color Doppler sonography and MRI plain scan and DCE-MRI were applied in all cases. <p>RESULTS: Of 12 cases, 7 cases were males and 5 were females, with the duration of symptoms ranged from 3mo-20y, with an average course of 3.2y; 6 cases were the primary cases and 6 cases were relapse cases. All lesions involved one side of the orbit, of which 8 cases were in the left orbit and 4 in the right. Of 12 cases, there were 5 tumors in intraconal space, 3 in the extraconal space, and 4 in intra and extraconal space. Well-circumscribed lesions showed oval shape in 9 cases and the left 3 were irregular. 12 cases showed hypoechoic and 2 cases heterogeneous signal on color Doppler sonography; All cases had flow signals on CDFI, and showed arterial spectrum on PW. On T1WI, all lesions demonstrated isointense. On T2WI, 5 lesions showed hypointense, 3 lesions showed isointense and 4 lesions slight hyperintense, of which 3 lesions showed heterogeneous signal. After contrast enhancement, all cases demonstrated markedly enhancement, with homogeneous enhancement in 10 cases and heterogeneous enhancement in 2 cases. The time-intensity curves(TIC)of 7 cases exhibited a rapid washout pattern, and 5 cases a rapid plateau pattern on DCE-MRI. <p>CONCLUSION: Color Doppler sonography features of orbital SFT include hypoechoic and flow signals. Heterogeneous signals on T2WI, marked enhancement, and a rapidly enhancing and slow washout pattern TIC on DCE-MRI are the typical MRI features of orbital SFT.

Primary chondromyxoid fibroma of the orbit: An orbital mass with calcification

Primary orbital chondromyxoid fibroma is a rarely reported entity. A 34-year-old lady presented with painless, non-axial proptosis of the left eye of 6 months duration. Orbital imaging showed a supero-temporal mass with calcific foci and bone erosion. The mass caused globe compression resulting in choroidal folds. Anterior orbitotomy with complete mass excision was performed. The histopathology revealed a chondromyxoid fibroma. At 12-months follow-up, the patient is doing fine with no clinical recurrence. Chondromyxoid fibroma is an important differential diagnosis for bony orbital tumors.

Proptosis por Celulitis Orbitaria en paciente Pediátrico: reporte de un caso / Proptosis for Orbital Cellulitis in a Pediatric Patient: case report

Orbital cellulitis is an infectious disease that occurs most frequently in the pediatric age. The most common underlying factor for its development is ethmoidal sinusitis. The microorganisms associated with orbital infection are S. pneumoniae, S. aureus, H. influenzae and M. catarrhalis, whose therapeutic failure brings serious complications that include loss of vision, meningitis and intracranial infection. In the following case we want to capture the doctor's actions in this infectious pathology, the diagnoses we should rule out and antibiotic treatment. We report the case of a previously healthy 2-year-old male patient who consulted for an increase left bipalpebral volume, associated to impossibility of spontaneous opening of left eye and febrile peak of 39.4 ° C, with TAC of paranasal sinuses and orbit, where shows left medial intraorbital abscess, with preseptal compromise that determines left proptosis, deciding to start associated antibiotic therapy for orbital cellulitis with compromise preseptal. In view of the slow evolution, a study was started to rule out the associated tumor process, which is discarded, maintaining an antibiotic for 21 days with clinical improvement after these. Orbital cellulitis in the pediatric age should be diagnosed quickly and in a timely manner, since it is a medical emergency, it is a condition that requires hospitalization and management with systemic antibiotics.

Clinical and pathological observation of 455 cases of orbital soft tissue tumor / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To investigate the clinic opathological features of orbital soft tissue tumors.<p>METHODS: A retrospective analysis of 455 cases of orbital soft tissue tumors in our hospital from 2003-11/2018-11 were performed to observe the clinical features, pathological classification and pathological features of rare tumors.<p>RESULTS:All 455 patients with orbital soft tissue tumors, 421(92.5%)were benign tumors. The top 5 were 258 cases of cavernous hemangioma, 58 cases of capillary hemangioma, 16 cases of neurofibroma, 15 cases of fibroid, and 14 cases of schwannoma; 27 cases(5.9%)of intermediate type, including 23 cases of solitary fibrous tumor(SFT), 2 cases of low-grade malignant fibrous histiocytoma, 2 cases of low-grade mucinous neurofibroma; 7 cases(1.5%)of malignant type, 2 cases of orbital malignant SFT, 2 cases of orbital myeloid sarcoma(MS), mucinous liposarcoma(MLS), spindle cell undifferentiated sarcoma and peripheral primitive neuroectodermal tumor(PNET)1 case. Immunohistochemistry and molecular detection of some cases revealed that the application of new antibodies such as STAT6 and molecular detection techniques can improve the diagnostic accuracy.<p>CONCLUSION:Benign tumors account for the majority of orbital soft tissue tumors. Cavernous hemangioma in vascular tumors is the first, and there are few soft tissue tumor pathological types such as Erdheim-Chester disease(ECD)and granulomatous ossifying fibers. Tumors(POF), leiomyomas and myxomas; intermediate and malignant tumors are rare; pathological types such as SFT, MLS, spindle cell undifferentiated sarcoma and MS are often difficult to diagnose, easy to miss diagnosis and misdiagnosis, new immunity histochemical antibodies and molecular detection techniques can improve the accuracy of the diagnosis.

Curative effect of different surgical approaches for orbital tumors / 国际眼科杂志(Guoji Yanke Zazhi)

· AIM:To analyze the clinical effect of different surgical approaches for orbital tumors.· METHODS:A total of 52 patients (56 eyes) with orbital tumors who were admitted to the hospital from January 2012 to June 2017 were examined by brain MRI and orbital sectional CT scan to identify the relationship between tumors and surrounding anatomical structures.They were treated by orbitotomy,and the approaches included anterior conjunctival approach,lateral approach and combined medial and lateral approach.The removal of tumors,recovery of visual acuity,postoperative early complications and follow-up recurrence were statistically analyzed.· RESULTS:Among the 52 patients,there were 7 cases 8 eyes (14％) with residual tumors.The rate of residual tumors after anterior conjunctival orbitotomy (23％) was higher than that after lateral orbitotomy or combined medial and lateral orbitotomy (6％,7％;P＜ 0.05).The visual acuity was improved in 28 cases 30 eyes (54％),no change in 18 cases 19 eyes (34％) and decreased in 6 cases 7 eyes (13％).The incidence of early complications was 23％,including 2 cases 2 eyes with accidental loss of vision,2 cases 2 eyes with pupil changes,3 cases 3 eyes with ptosis and 5 cases 6 eyes with eye movement disorders,which mainly occurred in the elderly.The recovery of visual acuity and the incidence rates of complications (23％,19％,29％) of the three kinds of surgical approaches were close (P＞ 0.05).During the follow-up period,there were 3 cases 3 eyes (5％) with recurrence in the 52 patients,and the recurrence rate after combined medial and lateral orbitotomy (14％) was significantly higher than that after anterior conjunctival or lateral orbitotomy (4％,0;P＜0.05).· CONCLUSION:The appropriate surgical approach can successfully remove the orbital tumors,improve or stabilize the visual function,and reduce complications and recurrence.

A Case of Orbital Chondroma

PURPOSE: To report a case of orbital chondroma. CASE SUMMARY: A 15-year-old male presented with an 8-month history of left hypertropia. The best-corrected visual acuity was 20/20 in both eyes. The exophthalmometry showed no exophthalmos, with 13 mm in both eyes. There was a hard palpable mass at the superonasal orbit of the left eye. Orbital computed tomography showed a heterogenous soft tissue shadow at the superonasal orbit of the left eye, and orbital magnetic resonance imaging revealed a 25 × 16 × 20 mm well-defined mass with low signal intensity in the T1-weighted image, high signal intensity in the T2-weighted image, and heterogenous enhancement in the contrast enhanced T1-weighted image. The mass was surgically removed with anterior orbitotomy. A 27 × 17 mm well-capsulated lobular mass was found, and histopathological examination revealed hyaline cartilage and chondrocyte. The mass was diagnosed as a chondroma. CONCLUSIONS: A chondroma is a benign tumor, which usually occurs in long bones and the small bones of the hands and feet. It is very rare in the facial and pelvic bones. The sites of chondroma occurring in the head and neck include the ethmoid sinus and maxilla, but it is extremely rare in the orbit.

Whole Body Positron Emission Tomography/Computed Tomography (PET/CT) in the Evaluation of Ophthalmic Tumors / 华中科技大学学报（医学）（英德文版）

The clinical value of whole body positron emission tomography/computed tomography (PET/CT) as an imaging tool in diagnosis of ophthalmic tumors was investigated.The retrospective observational case series were performed on the patients with suspected ophthalmic tumors who underwent whole body PET/CT.The golden standard of diagnosis was the final pathological diagnosis or the results of long-term follow-up for patients without surgery/ biopsy.PET/CT findings were compared with the golden standard.The sensitivity,specificity,accuracy and positive likelihood ratio of PET/CT in the detection of ophthalmic tumors were calculated.The clinical application of PET/CT in different types of ophthalmic tumors was evaluated.The results showed that 30 patients (18 males and 12 females) with a mean age of 43.0 years (range 4-63 years) were collected.The mean sizes of orbital tumors and intraocular tumors were 26.8 mm×17.8 mm and 11.2 mm×6.1 mm,respectively.The overall sensitivity,specificity,accuracy and positive likelihood ratio of whole body PET/CT in ophthalmic tumors were 76.5％,71.4％,75.0％ and 2.67,and were 62.5％,100％ and 70.0％ in intraocular tumors,and those were 100％,60.0％ and 84.6％ in orbital tumors,respectively.PET/CT findings were applied to help make appropriate treatment options in 27 out of 30 patients (90.0％),and 12 (40.0％) patients changed the treatment strategy.False negative results in 4 cases and false positive results in 2 cases were observed in this series.It was suggested that PET/CT was an effective imaging modality in detecting,diagnosing and developing therapeutic schedule for patients with ophthalmic tumors.It was more sensitive and accurate for detecting orbital tumors than for detecting intraocular tumors.

Whole Body Positron Emission Tomography/Computed Tomography (PET/CT) in the Evaluation of Ophthalmic Tumors / 华中科技大学学报（医学）（英德文版）

The clinical value of whole body positron emission tomography/computed tomography (PET/CT) as an imaging tool in diagnosis of ophthalmic tumors was investigated.The retrospective observational case series were performed on the patients with suspected ophthalmic tumors who underwent whole body PET/CT.The golden standard of diagnosis was the final pathological diagnosis or the results of long-term follow-up for patients without surgery/ biopsy.PET/CT findings were compared with the golden standard.The sensitivity,specificity,accuracy and positive likelihood ratio of PET/CT in the detection of ophthalmic tumors were calculated.The clinical application of PET/CT in different types of ophthalmic tumors was evaluated.The results showed that 30 patients (18 males and 12 females) with a mean age of 43.0 years (range 4-63 years) were collected.The mean sizes of orbital tumors and intraocular tumors were 26.8 mm×17.8 mm and 11.2 mm×6.1 mm,respectively.The overall sensitivity,specificity,accuracy and positive likelihood ratio of whole body PET/CT in ophthalmic tumors were 76.5％,71.4％,75.0％ and 2.67,and were 62.5％,100％ and 70.0％ in intraocular tumors,and those were 100％,60.0％ and 84.6％ in orbital tumors,respectively.PET/CT findings were applied to help make appropriate treatment options in 27 out of 30 patients (90.0％),and 12 (40.0％) patients changed the treatment strategy.False negative results in 4 cases and false positive results in 2 cases were observed in this series.It was suggested that PET/CT was an effective imaging modality in detecting,diagnosing and developing therapeutic schedule for patients with ophthalmic tumors.It was more sensitive and accurate for detecting orbital tumors than for detecting intraocular tumors.

Orbitectomía en monobloque en un paciente con sarcoma neurogénico de la órbita / Single block orbitectomy in a patient with neurogenic orbital sarcoma

El sarcoma neurogénico es un tumor maligno que se origina en las células de Schwann de la vaina del revestimiento de los nervios periféricos y son poco frecuentes en la órbita. Se presenta un paciente de 23 años de edad, masculino, blanco, con antecedentes de neurofibromatosis tipo I, con desplazamiento anteroinferior del globo ocular izquierdo, dolor intenso y pérdida de la visión de 4 meses de evolución. Al examen oftalmológico se constató proptosis severa con descenso del globo ocular izquierdo, oftalmoplejia total, quemosis severa, hiperemia, opacidad corneal y aumento de volumen del párpado superior. En los estudios imagenológicos se evidenció lesión tumoral que ocupaba la totalidad del compartimiento orbitario sin infiltración de sus paredes óseas y con desplazamiento del globo ocular por fuera del reborde orbitario. Se realizó un abordaje quirúrgico combinado, y se logró una orbitectomía en monobloque con resección total del tumor y reconstrucción con colgajo rotado de músculo temporal ipsilateral. El estudio histopatológico informó la presencia de un sarcoma neurogénico de la órbita y se complementó con tratamiento adyuvante con radioterapia. El paciente se mantuvo controlado durante un año y a partir de esta fecha comenzó la aparición secuencial de varias lesiones a distancia(AU)

Neurogenic sarcoma is a malignant tumor that starts in Schwann cells of the peripheral nerves sheath and is rarely found in the orbit. Here is a 23 year old, male, Caucasian patient, with a history of Type-I Neurofibromatosis, and a left eye fore and lower side displacement of the left eyeball, intense pain and loss of vision for 4 months. A severe proptosis and the lowering of the left eyeball was detected during the ophthalmologic examination, as well as total ophthalmoplegia, severe chemosis, hyperemia, corneal opacity and increased upper eyelid volume. Imaging studies revealed a tumor lesion occupying the whole orbital compartment, with no bone wall infiltration, but causing the displacement of the eyeball out of the orbit border. A combined surgical approach was performed consisting in a single block orbitectomy with total tumor resection, as well as the reconstruction with the ipsilateral temporal muscle rotated flap. The histopathology study showed the presence of an neurogenic orbital sarcoma, so an adjuvant radiotherapy treatment was indicated. The patient was followed up for a year, after which the sequential occurrence of several lesions began(AU)

An Orbital Solitary Fibrous Tumor: Report of Two Cases with Different Presentation

Solitary fibrous tumour (SFT) is a rare spindle-cell neoplasm that can occur in the orbit. We report two cases of orbital SFT in a 35-year-old female and a 28-year-old male with different presentations. First patient presented with slowly progressive left lateral upper lid mass which is firm in consistency and non tender. Patient had non axial proptosis as the mass compressed the globe inferonasally. There was also funduscopy evidence of choroidal folds superotemporally. There is slight impairment of vision on the left eye comparing to right eye. Meanwhile, the second patient presented with a painless diffuse swelling of left upper eyelid. It was soft in consistency, non fluctuate and no opening or pus discharge noted. There is mild mechanical ptosis, however there was no proptosis and no evidence of compression into the globe nor visual impairment.Computed tomography (CT) imaging revealed a well circumscribed and contrast enhanced soft tissue mass intraorbital extraconal mass in both cases. However in the first case, the tumour was at the level of lacrimal gland with compression of the globe, while in second case, it was superior and posterior to the left lacrimal gland with no globe compression. Both patients underwent complete resection of their tumors. The histological findings showed alternating hypercellular and hypocellular areas composed of bland spindle cells with a fibrous stroma. The strong immunoreactivity for CD34 supported the diagnosis of orbital SFT. There was no recurrence at the 2nd and 3rd year follow-up visits for both patients. SFT should be considered as one of the differential diagnosis of an orbital tumor. The combination of CT scan, histologic findings and immunohistochemical staining provide accurate diagnosis. En bloc excision of the tumour is the mainstay of treatment.

A Case of Primary Orbital Peripheral T-cell Lymphoma with Panniculitis-like Features

PURPOSE: To report a case of complete remission of primary orbital peripheral T-cell lymphoma with panniculitis-like features after chemotherapy. CASE SUMMARY: A 57-year-old healthy female presented with periorbital swelling and symptoms of diplopia. The patient was first treated with high-dose systemic corticosteroids, however, symptoms persisted. Therefore, anterior orbitotomy with excisional biopsy was performed for diagnostic purposes. On microscopic examination, the excised mass showed localized dense lymphocyte infiltrates, and cytologic atypia was observed under a high-power field. On immunehistochemical examination, tumor cells were positive for CD3 and CD8 but negative for CD4, CD20 and CD56. Based on histopathological results, primary orbital peripheral T-cell lymphoma with panniculitis-like features was diagnosed. Additionally, molecular pathological testing was positive for Epstein-Barr virus. Subsequently, the patients underwent chemotherapy and complete remission was obtained. CONCLUSIONS: Peripheral T-cell lymphoma often manifests as systemic symptoms, including lymph node enlargement and B symptom. The primary form of the disease in an orbit is very rare, and has a poor prognosis with a high mortality rate because the disease quickly progresses. Herein, the authors report a rare case of a healthy patient without any past medical history who achieved complete remission of a fast-growing primary orbital T-cell lymphoma with no preceding systemic symptoms.

Central retinal artery occlusion following orbital tumor resection: Is rapid intervention effective.

A 52‑year‑old male patient presented at our hospital with unilateral proptosis and vision loss in his left eye. Imaging evaluations showed orbital tumor, so the patient underwent surgery. About an hour later after tumor removal, patient developed sudden vision loss and became no light perception. Fundus evaluation revealed central retinal artery occlusion (CRAO). The patient was treated immediately with ocular massage and anterior chamber paracentesis as well as systemic therapy with mannitol and intravenous administration of acetazolamide. After thirty minutes, he recovered perception to light and then hand motion and 2 h later, it was improved to 1 m counting finger. CRAO following orbital tumor has not been reported before. We recommend ocular examination in all patients that undergo orbital surgery immediately to 2–3 h after surgery.

A Case of Steatocystoma Simplex of the Orbit

PURPOSE: Steatocystoma is a cyst that originates embryologically from hair follicles and usually occurs on the face, chest, back and axillae as multiple lesions and rarely occurs as a solitary lesion. We experienced a case of steatocystoma simplex that developed in the orbit and present our case with a brief review of the literature. CASE SUMMARY: A 44-year-old male presented with a history of palpable mass in the right inferomedial periorbital area that had been slowly growing for a few months. Physical examination revealed a non-tender, soft and round mass. Magnetic resonance imaging of the orbit showed 2.0 x 2.0 x 2.0 cm-sized mass in the right inferomedial orbit. We performed excisional biopsy of the orbital mass using transconjunctival approach. The pathological diagnosis was steatocystoma. There was no local recurrence during the postoperative follow-up. CONCLUSIONS: We experienced a rare case of steatocystoma simplex that developed in the orbit. Base on our results, steatocystoma simplex should be considered when diagnosing an orbital mass.

A Case of Granular Cell Tumor of the Orbit

PURPOSE: To report a case of granular cell tumor of the orbit in a patient, complaining of foreign body sensation due to entropion. CASE SUMMARY: A 59-year-old man visited our clinic because of foreign body sensation in the right eye due to entropion. A white mass was noted at the right palpebral conjunctiva, and bulbar conjunctiva chemosis was seen. We performed excisional biopsy and capsulopalpebral fascia repair for lower lid entropion. Histopathological examination revealed a granular cell tumor. There were no complications after surgery. CONCLUSIONS: Granular cell tumor of the orbit is rare, but it should be considered in the differential diagnosis of a mass lesion causing entropion or diplopia.

A Case of Erdheim-Chester Disease with Diplopia

PURPOSE: We present a case of Erdheim-Chester disease (ECD) with diplopia. CASE SUMMARY: A 56-year-old woman came to the hospital with a 6-week history of diplopia on left lateral gaze. The right eye showed mildly limited adduction. Humphrey automated perimetry demonstrated inferior bitemporal quadrantanopia. Orbital and brain magnetic resonance imaging revealed well-defined orbital masses in both intraconal orbits with homogenous enhancement, as well as multiple masses of homogenous signal intensity in the brain. Systemic evaluation showed involvement of the long bones, and retroperitoneum, but no involvement of the heart, or lungs. Incisional biopsy of the right orbital mass was performed. Histopathological examination showed numerous lipid-laden histiocytes and few multinucleated Touton giant cells. Immunohistochemical staining showed positivity for CD68, but negativity for CD1a, and ECD was therefore diagnosed. The patient received treatment with radiation therapy and interferon-alpha, but died due to sepsis secondary to urinary tract infection after 2 months. CONCLUSIONS: Except exophthalmos, diplopia may be the only initial symptom of an orbital mass. Although rare, the possibility of ECD should be considered in the differential diagnosis of both retrobulbar and orbital masses with diplopia.

Anatomy and preliminary clinical applications of endoscopic endonasal approach to optic canal and orbit / 解剖学报

Objective To provide the endoscopic anatomic basis and anatomic parameters for endoscopic surgical therapy on orbital lesions , and to analyze the advantages and key points of this surgical approach .Methods Five fresh adult heads were used in this experiment .Endonasal thanslamina approach and endoscopic technique were applied to observe important anatomic marks while intraoperative medicalization of the medical rectus muscle was applied to observe the exposure and positioning of important structures and trends of the optic canal and intra orbit .Results Uncinate process was at the lower front corner of middle nasal concha; ethmoidalis bulla was behind the uncinate process , and ethmoidei sinus was reachable after an incision was made on ethmoidalis bulla;anterior ethmoidal artery and posterior ethmoidal artery were the important anatomic landmarks of the inner ethmoidei sinus;optic canal prominence , carotid artery prominence and OCR were the important anatomic landmarks of the inner sphenoid sinus; lamina papyracea was at the lateral wall of ethmoidei sinus , and orbital contents were approacchable after lamina papyracea was cut off ;inside orbit , the optic nerve was approachable through the gap between the medial rectus muscle and inferior rectus muscle .The ophthalmic artery of 9 out of 10 sides of the specimens was originated from the supraclinoidal segment of the internal carotid artery while the remaining one was originated from the cavernous segment of internal carotid artery .There were 7 sides in which ophthalmic artery was located at the inferior lateral of the optic nerve;there were 2 sides in which ophthalmic artery was located at the inferior of the optic nerve; the remaining one was located at the inferior medial of the optic nerve .Conclusion The endoscopic endonasal thanslamina approach can sufficiently expose the optic nerve and the structures in the medical space of the orbit.Uncinate process, ethmoid bulla, anterior ethmoidal artery, posterior ethmoidal artery and posterior ethmoid sinus are the important landmarks of the endoscopic endonasal thanslamina approach .Optic canal prominence , internal carotid artery prominence and OCR are the important landmarks for optic canal decompression .Ophthalmic artery , orbital branches , anterior ethmoidal artery , posterior ethmoidal artery , internal carotid artery are the important vessels . Medialization of the medial rectus muscle is effective to approach the orbital anatomical structures .